Pattern of pressure gradient alterations after venous sinus stenting for idiopathic intracranial hypertension predicts stent-adjacent stenosis: a proposed classification system.

OBJECTIVE: Venous sinus stenting (VSS) is a safe and effective treatment for idiopathic intracranial hypertension (IIH) with angiographic venous sinus stenosis. However, predictors of stent-adjacent stenosis (SAS) remain poorly defined. METHODS: We performed a retrospective review of 47 patients with IIH and intracranial venous stenosis who underwent VSS with pre- and post-stent venography. Patient characteristics, treatments and outcomes were reviewed. Changes in pressure gradient after VSS were classified according to pattern of gradient resolution into types I-III. RESULTS: Type I gradient resolution, in which mean venous pressure (MVP) in the transverse sinus (TS) decreases towards MVP in the sigmoid sinus (SS), occurred in 18 patients (38.3%). Type II gradient resolution pattern, in which SS MVP increases towards that in the TS, occurred in 7patients (14.9%). Type III pattern, in which MVP equilibrates to a middle value, occurred in 22patients (46.8%). SAS occurred in 0%, 28.6%, and 22.7% of patients in types I, II and III, respectively. Compared with patients with type I gradient resolution, SAS was more common in those with type II (p=0.0181) and type III (p=0.0306) patterns. CONCLUSIONS: The pattern of change in the trans-stenosis venous pressure gradient may be predictive of SAS and is a useful tool for classifying the response of the venous obstruction to stenting. A type I pattern appears to represent the ideal response to VSS. Some patients with type II and III changes, particularly if they have other predictors of recurrent stenosis, may benefit from longer initial stent constructs.

Pediatric Pseudotumor Cerebri Syndrome: Diagnosis, Classification, and Underlying Pathophysiology.

Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS can occur in the pediatric and adult populations and, if untreated, may lead to permanent visual loss. In this review, discussion will focus on PTCS in the pediatric population and will outline its distinct epidemiology and key elements of diagnosis, evaluation and management. Finally, although the precise mechanisms are unclear, the underlying pathophysiology will be considered.

[Idiopathic intracranial hypertension as a cause of headache]. / Idiopatisk intrakranial hypertensjon.

Idiopathic intracranial hypertension (IIH) is characterised by increased intracranial pressure with normal cerebrospinal fluid, and no evidence of space occupying process, meningeal pathology or venous thrombosis. The condition is associated with obesity, especially in women of childbearing age. IIH is a rare but serious cause of headache, and constitutes a differential diagnosis for sudden-onset headache, particularly if the patient has visual disturbances not related to migraine and reports pulsatile tinnitus, cranial nerve palsy or radiculopathy.

Baseline visual field findings in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT).

PURPOSE: To characterize visual field (VF) loss at the baseline visit and to evaluate VF quality control (QC) procedures in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). METHODS: The Visual Field Reading Center (VFRC) evaluated 660 baseline VFs (1320 hemifields) from 165 enrolled patients. Three readers independently classified each superior and inferior hemifield and identified any abnormalities. A subset (20%) of the hemifields was reread to evaluate within- and between-reader agreements. The QC system addressed test parameters, patient data, and shipment errors. RESULTS: The majority (60%) of the baseline hemifields consisted of localized nerve fiber bundle-type VF loss. Approximately one-third (31.5%) of all the classifications consisted of partial arcuate defects combined with an enlarged blind spot, making this the most common type of hemifield classification. Inferior hemifield loss was greater than superior loss for both study and nonstudy eyes. Reader agreements were >90% for both inferior and superior hemifields for two out of three readers. Test-retest reliability agreement for individual readers was 95% for both hemifields. There were few QC errors with only 5.48 error points per 100-point VF. CONCLUSIONS: The most common type of IIHTT baseline hemifield abnormality was a localized nerve fiber bundle-like defect. Localized inferior hemifield loss was more common than superior hemifield loss. Quality control and within- and between-reader agreement were excellent for the IIHTT (ClinicalTrials.gov number, NCT01003639).

Headache in patients with idiopathic intracranial hypertension: a pilot study to assess applicability of ICHD-2 diagnostic criteria.

Headache is one of the most common symptoms of idiopathic intracranial hypertension (IIH). The aim of this study was to investigate the applicability of the diagnostic criteria for "Headache attributed to IIH" included in the current classification of headache disorders, particularly as far as the main headache features. A consecutive clinical series of IIH patients with demonstration of increased intracranial pressure by lumbar puncture in the recumbent position were enrolled. Among a total of 22 patients, headache was reported by 14. The proportion of patients reporting the main headache features required by diagnostic criteria were: 93 % for daily or nearly-daily occurrence; 71.5 % for diffuse/non-pulsating pain; 57 % for aggravation by coughing/straining. Thus, these three headache features, at least one of which is required for diagnosis of headache attributed to IIH, were present in the vast majority of our sample, suggesting that their inclusion should be regarded as appropriate. The analysis of our results may suggest possible changes in the current ICDH-2 criteria for headache attributed to IIH, based on the following considerations: the existence of remarkable differences as far as the relative frequency of each headache feature; the fact that diffuse and non-pulsating pain-included in the current classification as a single requirement-were not always found together; the high frequency of migrainous associated symptoms (nausea or photophobia-phonophobia were present in 71.5 % cases).

Evaluation of sixteen children with pseudotumor cerebri.

Pseudotumor cerebri (PTC) is a clinical condition characterized by signs and symptoms of increased intracranial pressure, such as headache and papilledema. A total of 16 patients diagnosed with PTC [12 (75%) female; 4 (25%) male] were included in the study. The age of onset of symptoms was 123.4 +/- 34.3 months (range: 60-168 months). Obesity was found in four (25%) of them. Two patients had venous sinus thrombosis, two had corticosteroid withdrawal, and one had posttraumatic PTC. The most common symptom was headache, recorded in 93.8% of the patients. All patients were treated medically. Two patients in our group also required a lumboperitoneal shunt. In conclusion, PTC in children is rare. Both papilledema and sixth nerve palsy resolved rapidly with treatment. However, children can sustain loss of visual field and visual acuity despite treatment.

Idiopathic intracranial hypertension within the ICF model: a review of the literature.

Idiopathic intracranial hypertension (IIH) is an unusual disease, seen most often in women of childbearing age who are obese. If left untreated, IIH can cause chronic pain and blindness. Although IIH has been recognized by healthcare providers since the late 1880s, the cause is still not known and risk factors remain unclear. Treatment has not changed over the years. While professionals struggle to define, describe, and successfully treat IIH, persons with the disease are struggling to cope. Internet support group communications relate numerous personal stories of frustration, depression, pain, anxiety, and disability. The World Health Organization's International Classification of Functioning, Disability and Health (ICF) model provides an appropriate framework through which to view what is known and what is yet to be discovered about IIH. The ICF model was designed to complement the International Statistical Classification of Diseases and Related Health Problems, looking beyond mortality and disease by describing how people live with their health conditions. Applying this framework to IIH reveals many opportunities for nursing research within the ICF domains of health condition, body function and structure, ability and participation, and environmental and personal factors.

Clinical features of late-onset pseudotumor cerebri fulfilling the modified dandy criteria.

OBJECTIVES: To characterize the clinical features of patients with pseudotumor cerebri (PTC) fulfilling the Modified Dandy Criteria who were diagnosed at or after the age of 44 years. METHODS: We reviewed the medical records between 1987 and 1999 of 14 patients at a single institution who were diagnosed as having PTC at 44 years of age or older according to the Modified Dandy Criteria: neurologic manifestations attributable to generalized increased intracranial pressure, elevated cerebrospinal fluid pressure with normal cerebrospinal fluid composition demonstrated by lumbar puncture, and normal or small ventricles demonstrated by neuroimaging. We documented presenting symptoms and signs, significant medical conditions, and visual field follow-up. RESULTS: There were nine women and five men. Nine patients (64%) were obese. Five patients (36%) were asymptomatic. None presented with headache alone. Four patients (29%) had an identifiable cause of intracranial hypertension, including two with transverse sinus thrombosis, one with severe chronic obstructive pulmonary disease and cor pulmonale, and one with corticosteroid withdrawal after prolonged administration. During a median follow-up of 2 years of 12 patients, visual fields remained stable in 8, improved in 3, and worsened in 1. CONCLUSIONS: In comparison with patients who have idiopathic intracranial hypertension, our small series of 14 patients diagnosed after the age of 44 years were more often men, were less often obese, were less symptomatic, and had identifiable causes of intracranial hypertension in a substantial minority (29%). The visual prognosis in this age group is generally good. Because the nonidiopathic causes of PTC would be overlooked by adhering to the Modified Dandy Criteria, we propose a modification that excludes patients who have dural venous sinus disease demonstrated on magnetic resonance imaging and those who may be exposed to medications or toxins or have systemic disorders that are known to increase intracranial pressure.

A new classification and a synergetical pattern in intracranial hypertension.

Intracranial hypertension develops from the initial cerebral effect of increased intracranial pressure and becomes symptomatical; then it acquires its individuality, surpassing the initial disease. The intracranial hypertension syndrome corresponds to the stage at which the increases in intracranial pressure (ICP) can be compensated and the ICH disease is in its acute form, equivalent to a decompensated ICH syndrome. Based on the etiopathogenesis of intracranial hypertension, a new classification is proposed: parenchymatous intracranial hypertension with an intrinsic cerebral cause; vascular intracranial hypertension, which has its etiology in disorders of the cerebral blood circulation; and essential or idiopathic intracranial hypertension, the former pseudotumor cerebri, an incomplete ICH syndrome. A synergetical pattern of the ICH is based on the relation between ICP and the period of high-pressure action: the critical pressure--time fluctuation causes the autoregulation of the cerebral blood flow to decrease or determines the brain herniation. The decompensation is a state of instability and appears when the intrinsic ratio of pressure--time fluctuation is changed: the high ICP lasts longer than the corresponding normal ICP, or the ICP is higher than the one that normally lasts the same period of time.

The pseudotumor syndrome. Disorders of cerebrospinal fluid circulation causing intracranial hypertension without ventriculomegaly.

We report a series of eight cases that show a close resemblance to, but are not identical with, pseudotumor cerebri (PTC) as normally defined. The majority of these cases are characterized by raised intracranial pressure without ventriculomegaly. They include two cases of cranial venous outflow obstruction in which clinical or radiologic abnormalities precluded the diagnosis of PTC proper (cases 1 and 2); one case of chronic meningitis in which an abnormal cerebrospinal fluid (CSF) composition precluded the diagnosis of PTC (case 3); two cases without either papilledema or a measured increase of CSF pressure, which in other respects, particularly in response to treatment, resembled PTC (cases 4 and 5); and three cases of what is thought to represent an infantile form of PTC (cases 6 through 8). The purpose of the analysis of these cases is twofold. First, it is argued that these cases throw light on the mechanism of PTC itself, supporting a concept of a disturbance of CSF circulation in this condition, and that they are themselves illuminated by considerations of typical PTC. Second, the cases are used to frame a proposed classification of the pseudotumor syndrome aimed at broadening the diagnostic criteria applied currently to PTC. It is suggested that the pseudotumor syndrome has a single underlying mechanism (disturbed CSF circulation) and that recognition of this mechanism not only clarifies the pathophysiologic processes of PTC but also has important diagnostic and therapeutic implications.

[Benign intracranial hypertension. A clinical, pathophysiological and diagnostic study (author's transl)]. / L'hypertension intracrânienne bénigne. Etude clinique, physiopathologique et nosographique.

Sixteen unpublished observations of Benign intracranial hypertension were reviewed from a clinical, aetiological and prognostic standpoint. The hypothesis that this affection could be caused by some disturbance of the C.S.F. resorption was assessed using an experimental tests battery allowing the measurement of the main factors involved in C.S.F. resorption. Our patients presented with a pure, solitary state of intra-cranial hypertension, of variable duration, capable of returning. The vital outcome was always favourable, but several severe and protracted cases were marked by a definitive visual damage. The visual risk, often underlined in the literature, requires a careful attention and eventually needs some effective treatment including C.S.F. diversion. A disorder of C.S.F. absorption could be demonstrated in most of our observations and appears to account for the principal features of Benign intracranial hypertension, including the lack of ventricular enlargement. The absorption disorder resulted either from the reversion of the pressure gradient between the C.S.F. and the venous sinuses when a dural sinus was obstructed, - or from an elevation of the resistance to flow when the sinuses were patent, thus suggesting some structural alteration of the arachnoid villi. However, for lack of histological control, such an alteration remains hypothetical, and a primary brain edema probably yield a similar a pathophysiological pattern. Finally, an attempt is made to classify the various aetiological factors encountered in Benign intracranial hypertension according to the previous pathogenic discussion.